CONFIRMED - I have Pulmonary Hypertension :(

Since my last post things have just gone down hill again and the past few months have been a great struggle both emotionally and physically. As I mentioned last time I was due to be admitted to Hammersmith Hospital to undergo some further tests in order to confirm Prof Heaton's hunch that I may have Pulmonary Hypertension and to see if how significant it is which he also thinks it has already reached a significant stage as my symptoms would suggest.Also I have noticed that every week I am finding it more and more difficult to breathe and it takes a lot out of me just to do the normal everyday activities such as making my bed, doing household chores and even just getting up to go to the toilet or dress myself. I have to do most things sitting down and I can't lie flat as I can hardly breathe when lying down, so I have to sleep propped up with loads of pillows or sitting down which makes sleeping very difficult and uncomfortable and its not like I don't have trouble sleeping anyway due to my insomnia. Actually I can't remember when was the last time I actually had a good quality sleep and when I am in hospital the nurses always ask me if I ever sleep.

During my elective admission at Hammersmith Hospital (Imperial) I underwent numerous procedures and tests including a cardiac catheter, walking, breathing and bicycle tests over three days  and was due to be discharged as soon as the results came back and my consultant had decided as to what the best treatment plan is for me and discussed my options. However, in classic Maribel style on the day of my discharge I woke up very confused, intense jerking and my speech was very slurred so when my consultant came on her ward rounds and intended to discuss my results and her plans for my treatment she was shocked to find me in the state I was so she immediately rang the Liver team at Kings to try and get me transferred as they do have a Liver unit within the hospital and due to my complicated medical history she said she didn't want to interfere with my Liver. However, as usual Kings never have free beds so was put on the waiting list which was quite long therefore, I explained how thy usually treat me whenever my encephalopathy comes on so they just loaded me with lactulose and phosphate enemas over the next few days until I was completely back to normal and by that time a bed still had not become available at Kings so instead I was discharged the following Monday. My initial four day planned admission turned into eight days.

Due to my encehalopathy episode unfortunately my PHT consultant suggested it was best not to start the new drug treatment until I was completely cleared of the enceph as it could mask any reactions and side effects of the new drug. as soon as I was no longer confused and could talk properly she sat down with me and discussed my tests results and the treatment available and what she suggests was the best option for me would be.

RESULT based on the following tests:

1. Metabolic exercise test
2. Lung function
3. Right heart catheterisation
4. V/Q scan & CPTA
5. Autoimmune serology
6. Viral serology
7. Thyroid function test
8. Echo-cardiogram

Diagnosis consistent with porto-pulmonary hypertension. Treatment with vasodilators was discussed and decided with my consultant.

On December 11th I was admitted for a day case to begin treatment on the vasodilators a medication used to improve and control the symptoms of Pulmonary Hypertension as it lowers the pressure around the heart allowing me to breathe easier. It is not a cure but will hopefully improve my quality of life. Although the treatment is non-invasive as it is only taking a pill three times a day it was still important that it was done as a day case in order to monitor me for a few hours after taking the firs dose as one of the side effects is that it can lower blood pressure significantly and if that is the case then they would have to find an alternative. Also due to my history of drug allergies and reactions to new drugs was more reason for me to be monitored. So they started me on the normal dose of 20mg and after an hour my BP dropped significantly  and after another hour it dropped again and so by this point I was getting worried as the nurse said that if the next one has gone down more then they would have to inform my consultant and that I would possibly need to be admitted overnight which I was praying would not happen. Fortunately for me the next reading had not changed much. It didn't increase but  did not go down too much so I just about got away with it and was allowed to go home. They gave me one month supply and said that it will take maximum of 6-8 weeks for it to start taking affect  and if by that time I don't feel any different then they will increase my dose as the medication I am on for my encephalopathy reduces the affect of the sedenafil (vasodilator).

t has been 2 weeks since I started the new drug and I haven't felt any different which I know is normal as they did specifically say 6-8 weeks. I really hope this treatment works as my quality of life is getting worst by the day and on top of my insomnia as to why I can't sleep , now I can't lie down flat as I cant breathe so I
have to either sleep sitting up right or my head propped up on loads of pillows. Also just doing the little everyday things is so difficult, for exaple washing just one dish or cooking something takes up so much energy and I feel exhausted as well as getting up during the night to go to the toilet which id like right next to my bedroom gets me so breathless by the time I get back into bed. You would think I've just ran up 10 flights of stairs by the way I am puffing and gasping for breath. I'm so fortunate that I got this ground floor flat because I can no longer  go up and down stairs so when I go to mum's or visit family and friends it takes me about 15-20 mins to get up 2 flights of stairs and have to stop on every level to sit down and catch my breath before I can go up the next set. Being such an independent and outgoing person I am getting so frustrated not being able to do things for myself and going out is not as much fun any more cos of all the aches and pains and having to stop all the time. It is really getting to me now and getting me more depressed. When is it all going to end? I often ask myself!